CRESTVIEW — When an ultrasound uncovered more than her baby’s gender, 29-year-old Ashley Hewett’s life became a living nightmare. 

“This was supposed to be an exciting appointment to find out we were having a boy and then now it’s deeper than that,” she said.

The ultrasound revealed that the left ventricle of her son’s heart was significantly smaller than the right, but Hewett wasn’t too concerned when she visited doctors at Sacred Heart Hospital in Bluewater. 

“They didn’t give me a lot of information when they referred me to get checked,” she said. “When I was referred they just said, ‘well, it looks like one side of the heart must be smaller, so you might want him to get checked just to be sure.’”

She added, “I didn’t have the impression that it was anything to worry about.”

The following week, a high-risk specialist at Sacred Heart Hospital in Pensacola diagnosed her son with hypoplastic left heart syndrome, a rare, congenital heart defect that affects normal blood flow because the left side of the heart cannot pump oxygen-rich blood throughout the body.

According to the Centers for Disease Control and Prevention, HLHS affects about one out of every 4,344 babies, or roughly 960 babies born in the U.S. each year. Babies born with this birth defect often have bluish, ashen skin and a weak pulse. And although a cause for the disease is still largely unknown, it’s thought to be genetic in origin. 

Hewett’s son, David, arrived five weeks prematurely on April 23. Shortly thereafter, doctors determined that he would need a new heart because his tricuspid valve was leaking. “At first we felt like we were going to have a baby that might not live and this was our first baby,” Hewett said. “We almost felt hopeless.”

Generally, babies born with HLHS normally undergo a three-step surgical procedure before a transplant is required:

Step 1: The Norwood Operation — takes place in the first few weeks after birth. Surgeons rebuild the aorta then reconnect it to the heart’s right ventricle, allowing the right ventricle to efficiently pump blood to both the body and the lungs.  If a baby survives this procedure their odds of survival are said to increase. According to an article in the Journal of Pediatric Health titled “Hypoplastic left heart syndrome: Prognosis and management options,” the survival rate for babies who have the Norwood Operation ranges from 47 percent - 85 percent.

Step 2: Hemi-Fontan and a bi-directional Glenn procedure — takes places when the baby is between four and 12 months old. In this procedure, surgeons reroute the circulation so the superior vena casa drains into the right pulmonary artery. Hospital survival rates for the Hemi-Fontan procedure are 94 percent – 98 percent.

Step 3: The Fontan operation — takes place when the child is between 18 months and 2 years old. According to an article by the University of California San Francisco’s Department of Pediatric Cardiothoracic Surgery, the Fontan procedure connects the inferior vena cava to the pulmonary artery by making a channel through or just outside of the heart to direct blood to the pulmonary artery.  Survival rates for the Fontan procedure are 86 percent – 94 percent.

Hewett's baby, David, who had to forgo the aforementioned procedure because of a leaking tricuspid valve, was placed on the heart transplant list a few weeks ago. Since then, a lack of blood flow has caused his organs to partially shut down. The Hewetts can only hope that a heart becomes available quickly because longer wait times on the heart transplant list have been linked to higher infant mortality rates.

“According to the doctors last month, he wasn’t going to make it,” said Hewett. “They took him off the transplant list and gave us options to basically watch him die.” But in the last week or so, there has been a bit of a turnaround for David, whose condition has improved somewhat since he was taken off medication that was adversely affecting his liver. 

“He is showing all the doctors that he’s moving in the right direction and the doctors keep coming in and commenting on how much better he looks, they are really impressed, they almost can’t believe it,” said Hewett. “I think one doctor said, ‘this is suspiciously good.’”

Ashley and her husband, Chuck, also 29, have been staying at the UF Health Shands Hospital in Gainsville for the last two months after moving out of the Ronald McDonald House. “About a month ago after things started to look worse we moved into his hospital room and have been staying by his side,” she said.

Survivors of HLHS require constant medical attention, and since their hearts do not function at full capacity, those born with HLHS who make it into adulthood cannot exercise, and/or stand for long periods of time without having to sit; and often, most need more than one heart transplant.

Some of the longest-living survivors are now in their late 20s and early 30s.